Objective: This study evaluates the 24-month follow-up for the NICHD Neonatal Research Network (NRN) Inositol for Retinopathy Trial. Study design: Bayley Scales of Infants Development-III and a standardized neurosensory examination were performed in infants enrolled in the main trial. Moderate/severe NDI was defined as BSID-III Cognitive or Motor composite score <85, moderate or severe cerebral palsy, blindness, or hearing loss that prevents communication despite amplification were assessed. Results: Primary outcome was determined for 605/638 (95%). The mean gestational age was 25.8 ± 1.3 weeks and mean birthweight was 805 ± 192 g. Treatment group did not affect the risk for the composite outcome of death or survival with moderate/severe NDI (60% vs 56%, p = 0.40). Conclusions: Treatment group did not affect the risk of death or survival with moderate/severe NDI. Despite early termination, this study represents the largest RCT of extremely preterm infants treated with myo-inositol with neurodevelopmental outcome data.

Background/aims To report the long term outcomes of photorefractive keratectomy (PRK) for the treatment of hyperopia associated with purely refractive accommodative esotropia. Methods Retrospective chart review of 40 patients age 17–39 who underwent PRK to eliminate their dependence on glasses. Pre and post-operative best spectacle corrected visual acuity (BSCVA), uncorrected visual acuity (UCVA), refractive spherical equivalent (SEQ), ocular alignment, and stereoacuity were reviewed. Results Forty patients (80 eyes) with mean age 27.9 years were treated for a mean pre-operative SEQ of +3.06D hyperopia. The mean final post-operative SEQ was +0.06. Pre-operative BSCVA was 0.04 logMAR, and did not change post-operatively. Mean UCVA significantly improved from 0.30 logMAR preoperatively to 0.08 logMAR post-operatively. Mean pre-operative esotropia at distance and near was 18.6 PD. All patients were orthophoric without correction at the one month, one year, and final post-operative evaluations. Visual acuity, refractive error and alignment remained stable after the one year post-operative examination. Stereoacuity was unchanged in 80% of patients postoperatively. There were no complications. Conclusion PRK can be used to treat low to moderate hyperopia associated with purely refractive accommodative esotropia in young adults.

Objective: To design a simple matching acuity test based on hand gestures that is minimally dependent on familiarity with symbols and letters. The visual acuity results obtained from children using the Handy Eye Chart™ were compared with results obtained with the Early Treatment Diabetic Retinopathy Study (ETDRS) chart. Design: Evaluation of diagnostic test or technology. Participants: 60 children between 6 and 16 years of age were recruited consecutively from the Pediatric Ophthalmology section of the Emory Eye Center. Methods: Monocular visual acuity was tested using both the new eye chart and the ETDRS chart, alternating the order of administration between subjects. Testing was performed on the subject’s eye with the poorest acuity. Main Outcome Measures: Outcome measures were monocular logarithm of the minimum angle of resolution (logMAR) visual acuity scores for each chart. Results: The acuities were shown to have a strong linear correlation (r = 0.95) and a mean difference in acuity of −0.03 (95% confidence interval −0.05 to −0.01) logMAR, equivalent to approximately 1.5 letters, with the new eye chart underestimating the vision as determined by the ETDRS chart. The 95% limits of agreement were ±1.6 lines. Conclusion: The present study supports the validity of the new eye chart as a measure of visual acuity in pediatric patients ages 6 to 18 with vision ranging from 20/16 to 20/200.

Purpose: To determine the value of serial personal photographs in timing the onset of unilateral cataracts in children over 6 months of age. Methods: Personal photographs from 11 children with unilateral cataracts who underwent CE and IOL implantation when ≥ 6 months of age were reviewed. Photographs were evaluated for changes in the red reflex which might indicate the presence of a cataract. Results: The children underwent cataract surgery at a mean age of 40.3 months. They were followed for a mean of 33.2 months. Nine children were diagnosed as having an acquired cataract by photographic review documenting a previously normal red reflex. The visual acuity in the affected eye of 4 of these 9 children improved to ≥ 20/60. Cataracts were visible on photographs prior to clinical diagnosis in 5 patients, from 0.5 to 22 months prior to clinical diagnosis, but visual outcomes were not correlated with the photographically documented duration of the cataract prior to treatment. Photographs were not helpful in timing the onset of cataract in two children due to the poor quality of the images. Conclusions: Serial personal photographs are helpful in determining whether cataracts are acquired. However, they have limited usefulness in predicting the visual outcome presumably because other factors such as compliance with patching therapy are also critical in achieving good visual outcomes.

Purpose: To report the outcomes after cataract extraction in eyes previously treated for retinoblastoma. Methods: We reviewed the medical records of consecutive patients treated with cataract extraction at our institution for radiation-induced cataract following retinoblastoma treatment between 1992 and 2007. Results: Twelve eyes of 11 patients were included. The median age at diagnosis of retinoblastoma was 7.4 months. The median quiescent interval before cataract surgery was 34.6 months (range: 17 - 240 months). All patients underwent limbal-based extracapsular cataract extraction with scleral-tunnel wound construction. Anterior vitrectomy and posterior capsulotomy were performed in seven eyes; an intraocular lens was placed in 10 eyes. A laser capsulotomy was subsequently performed in five eyes. No postoperative complications occurred. One patient underwent a vitrectomy for vitreous hemorrhage five years after cataract extraction. The median follow-up after surgery was 6.01 years (range: 1.1 - 12.3 years). Final visual acuity was between 20/20 and 20/60 in six eyes, between 20/70 and 20/200 in two eyes, and worse than 20/200 in four eyes. Every patient with documented preoperative vision (10 eyes) had improved vision at final examination. No intraocular recurrences or metastases occurred. Two patients developed secondary rhabdomyosarcomas, one of whom succumbed to their disease. Conclusions: Limbal-based cataract extraction in our series was not associated with tumor recurrence or metastasis. The optimal quiescent interval is not known, however, no tumor recurrences occurred in our series when a minimum 17 month quiescent period was achieved. Visual improvement was noted in every patient, although several patients only experienced modest gains.

Background: Horizontal strabismus due to a weak rectus muscle can be treated with an augmented Hummelsheim procedure, in which both vertical rectus muscle tendons are split, resected by 4 mm, and reattached to the sclera adjacent to the weak rectus muscle. Compared with vertical rectus transposition, the procedure spares two ciliary vessels and does not require placement of augmentation sutures. In this study, we evaluated binocular alignment and ocular motility in patients with abducens nerve palsy treated with an augmented Hummelsheim procedure. Methods: The medical records of consecutive patients with complete abducens nerve palsy who underwent the augmented Hummelsheim procedure, usually combined with medial rectus muscle recession, were retrospectively reviewed. Binocular alignment, ocular motility, and complications were analyzed. Results: Ten patients (age range, 12-57 years) met inclusion criteria for the study, of whom 9 of 10 had simultaneous medial rectus recession. Follow-up ranged from 1 week to 24 months. The augmented Hummelsheim procedure improved esotropia from 43Δ ± 5 Δ preoperatively to 6Δ ± 7 Δ postoperatively (P < 0.0001) and reduced abduction deficits from -4 to -3 (P < 0.0001). One patient with coexisting oculomotor nerve palsy developed consecutive exotropia; 2 had induced vertical deviations. There were no cases of anterior segment ischemia. Conclusions: The augmented Hummelsheim procedure combined with medial rectus muscle recession reduced mean primary position esotropia and improved abduction in patients with complete abducens nerve palsy.

Purpose: To evaluate an alternative retinopathy of prematurity (ROP) screening system that identifies infants meriting examination by an ophthalmologist in a middleincome country. Methods: The authors hypothesized that grading posterior pole images for the presence of pre-plus or plus disease has high sensitivity to identify infants with type 1 ROP that requires treatment. Part 1 of the study evaluated the feasibility of having a non-ophthalmologist health care worker obtain retinal images of prematurely born infants using a non-contact retinal camera (Pictor; Volk Optical, Inc., Mentor, OH) that were of sufficient quality to grade for pre-plus or plus disease. Part 2 investigated the accuracy of grading these images to identify infants with type 1 ROP. The authors prospectively recruited infants at Chulalongkorn University Hospital (Bangkok, Thailand). On days infants underwent routine ROP screening, a trained health care worker imaged their retinas with Pictor. Two ROP experts graded these serial images from a remote location for image gradability and posterior pole disease. Results: Fifty-six infants were included. Overall, 69.4% of infant imaging sessions were gradable. Among gradable images, the sensitivity of both graders for identifying an infant with type 1 ROP by grading for the presence of pre-plus or plus disease was 1.0 (95% confidence interval [CI]: 0.31 to 1.0) for grader 1 and 1.0 (95% CI: 0.40 to 1.0) for grader 2. The specificity was 0.93 (95% CI: 0.76 to 0.99) for grader 1 and 0.74 (95% CI: 0.53 to 0.88) for grader 2. Conclusions: It was feasible for a trained non-ophthalmologist health care worker to obtain retinal images of infants using the Pictor that were of sufficient quality to identify infants with type 1 ROP.

Purpose: To evaluate (1) the feasibility of nonophthalmologist healthcare workers (HCWs) obtaining images of sufficient quality for retinopathy of prematurity (ROP) screening using a Food and Drug Administration-approved portable, noncontact, narrow-field fundus camera (i.e., Pictor; Volk Optical, Inc, Mentor, OH) and (2) the accuracy of grading these images to identify infants in whom treatment-warranted (type 1) ROP developed. Design: Prospective cohort study. Participants: Infants undergoing routine ROP screening examinations (i.e., birth weight ≤1500 g, gestational age ≤30 weeks, or both, or selected infants with a birth weight of 1500–2000 g or gestational age >30 weeks and an unstable clinical course). Methods: We prospectively recruited infants undergoing ROP screening examinations at a community hospital. On the same day an ophthalmologist examined them, a trained HCW imaged their retinas using the noncontact camera. Two masked ROP experts graded these images remotely. We calculated both the percentage of gradable images (i.e., having at least 3 quadrants with sufficient image quality), as well as the accuracy of identifying infants in whom type 1 ROP developed. Main Outcome Measures: Percentage of gradable images and the sensitivity and specificity of each grader for identifying infants with type 1 ROP by grading for the presence of preplus or plus disease. Results: Ninety-nine infants were included. Overall, 92.4% and 94.2% of all infant imaging sessions were considered gradable by graders 1 and 2, respectively. Among gradable images, the sensitivity of both graders for identifying type 1 ROP by grading for the presence of preplus or plus disease was 100% (95% confidence interval [CI], 95%–100%), and the specificity was 91% (95% CI, 83%–95%) for grader 1 and 93% (95% CI, 86%–96%) for grader 2. Conclusions: It was highly feasible for trained HCWs to obtain digital retinal images of sufficient quality for ROP screening using a noncontact fundus camera. By grading for the presence of preplus or plus disease, graders identified infants in whom type 1 ROP developed with high sensitivity and specificity. The use of portable, noncontact retinal cameras by trained HCWs could increase our workforce in ROP screening and identify infants needing indirect ophthalmoscopy examinations by an ophthalmologist.

Waldenström macroglobulinemia (WM)/lymphoplasmacytic lymphoma (LPL) is a rare, chronic B-cell lymphoma with high heritability. We conduct a two-stage genome-wide association study of WM/LPL in 530 unrelated cases and 4362 controls of European ancestry and identify two high-risk loci associated with WM/LPL at 6p25.3 (rs116446171, near EXOC2 and IRF4; OR = 21.14, 95% CI: 14.40–31.03, P = 1.36 × 10−54) and 14q32.13 (rs117410836, near TCL1; OR = 4.90, 95% CI: 3.45–6.96, P = 8.75 × 10−19). Both risk alleles are observed at a low frequency among controls (~2–3%) and occur in excess in affected cases within families. In silico data suggest that rs116446171 may have functional importance, and in functional studies, we demonstrate increased reporter transcription and proliferation in cells transduced with the 6p25.3 risk allele. Although further studies are needed to fully elucidate underlying biological mechanisms, together these loci explain 4% of the familial risk and provide insights into genetic susceptibility to this malignancy.

PURPOSE: To compare the results of visual acuity testing in a population of deaf children using the Handy Eye Chart versus the Lea Symbols Chart and to compare testability and preference between charts. METHODS: A total of 24 participants were recruited at the Atlanta Area School for the Deaf. Visual Acuity was evaluated using the Handy Eye Chart and the Lea Symbols Chart. Patient preference and duration of testing were measured. RESULTS: The mean difference between the visual acuity as measured by each chart was -0.02 logMAR (95% CI, -0.06 to 0.03). Testing with the Handy Eye Chart was an average of 13.79 seconds faster than testing with the Lea Symbols Chart (95% CI, 1.1-26.47; P = 0.03). Of the 24 participants, 17 (71%) preferred the Handy Eye Chart (95% CI: 49%-87%; P = 0.07). CONCLUSIONS: The Handy Eye Chart is a fast, valid, and useful tool for measuring visual acuity in deaf children age 7-18 years. Additional research is needed to evaluate the utility of the Handy Eye Chart in younger children and deaf adults.